Duchenne Muscular Dystrophy

Assal Karimi-Rahjerdi

Special to the Ebbtide

I change my uncomfortable position on the hard metal bench, and look at him, his big brown eyes fixed on a spot as he goes up and down on a seesaw. It has been quite a while since the big, beefy looking muscles on his legs have drawn my attention. I can see the frustration on his face as he struggles to climb down on to the floor. "Mommy! Mommy! Tell them to stop running! Tell them to STOP!" His voice continues to get higher as his tears run down his face. He falls with every five steps that he takes, while trying to run after the other kids. It has just been a few months since he has been diagnosed with Duchenne Muscular Dystrophy, or DMD.

DMD, a hereditary degenerative disease of voluntary muscles, is considered the most prevalent form of childhood muscular dystrophy. The disorder is typically recognized from approximately age three to six years, and has a relatively rapid, progressive disease course. Duchenne Muscular Dystrophy is initially characterized by muscle weakness within the pelvic area, which may be followed by involvement of the shoulder muscles. With disease progression, muscle weakness and atrophy affect the trunk and forearms, and gradually progress to involve most major muscles of the body. Survival is rare beyond the early thirties, and by approximately age 10 to 12, most affected individuals require the use of a wheelchair.

Kamyar, turning six in February, cannot jump, run, or climb up stairs. As his parents pamper him excessively, he somehow knows that he is not like any other ordinary child, although beyond doubt, does not yet understand the meaning of a "fatal disease." You can't catch DMD; it's something that you are born with. DMD is not contagious, nor there are any environmental elements that we know of today, that cause it. It is simply an inherited disease. Kamyar's parents, however, do not want to accept that his disease is nobody's fault. Being cousins, they hold themselves responsible for his genetic disorder. They can't bear to watch their own flesh and blood grow up, knowing that they will lose him at a certain age. Kamyar's parents are going through denial.

Kamyar visits all kinds of doctors, hearing entirely different medication procedures. The impact of DMD can be minimized significantly by keeping the body as flexible, straight and mobile as possible. Although they have yet not found a way to stop or reverse the muscle degeneration of DMD, the scientists take three major treatment approaches to it.

First is the drug therapy, which is basically throwing drugs at it, hoping that they would slow it down for a while, without the serious damaging side effects, which can happen over an extended period of time.

The second approach is called gene therapy, which is done by sending a good gene into the defective cell on a virus, hoping that it will settle in, and take over for the defective gene.

The last method is cell transplant therapy, and is done by injecting a good cell into the affected muscle in an attempt to repair or regenerate the muscle.

Kamyar's visits to the doctors have not been really useful. "I do not want them to give up hope on their son. I will not tell his parents that his disease is deadly, as I believe that one day the cure will be found," said Dr. Soroush.

Some say that the disease is not fatal, not wanting to see the disappointment, while others stare at the floor and ask his parents to pray for his health or a miracle. He always screams with frustration when the doctors want to examine him. "Not my pants, tell him that I will not take off my pants", he shouts. As tears roll down his face and his mother tries to hide the big lump in her throat, he says, "Let's go home and eat. I promise I will eat as much as you want me to." After a lot of struggles, kicking, and fighting, they take him back home to get pampered again.

Disappointed with the general doctors, Kamyar's parents have now turned to some herbal treatments. The doctor that has suggested this procedure has given him oil, some type of dough, and a special kind of sneezing powder. The dough is to be spread over his head, the oil to be massaged on his muscles, and the sneezing powder to be held up to his nose so that he would sneeze several times a day. Losing the battle every time, Kamyar screams hateful words as his mom tries to cover his head with the dough. He says that he hates her for planting things in his head that stink.

As he rarely moves, exhaustion does not play an important role in his life. His daily schedule is fairly easy to follow. He turns on the television, takes out his drawing book and his black coloring pencil, and starts his daily routine. While he draws, nothing can distract him.He works on his drawing for hours, and mumbles television commercials to himself. He only draws with black pencils, and once, when I asked him the reason, he said that black is the most colorful of all pencils. His talented drawings bring tears to his parents' eyes. Every now and then he calls his mommy by his side, sticks his face to hers, and tells her how much he loves her. The afternoon is his most difficult time of the day, since he has to take a walk around the neighborhood with his mom, as a part of his treatment. He lives on the third floor of an apartment, and refuses to ask for help while going down the stairs. His mother walks closely behind him in order to watch out for him as he tries to take his little steps. After a dozen steps, she asks if he needs help, and he replies: "I am all grown up now. I am like Superman! I don't need your help, and stop looking at me. I don't like it when people look at me". Once he is outside, he would tell his mom his name of the day. "I am Spiderman today!" Then he wouldn't respond to his own name for the rest of the day, and will get very angry if people forget it. As he takes poor steps one after the other, he looks at the children run, and sighs. "I am the strongest! And everyone is scared of me!" he suddenly says out loud. At bedtime, his big eyes stay wide open and stare at his drawing book. He closes his eyes at sunrise and only sleeps for a few hours.

Kamyar does not know what awaits him. He is only living a nightmare. He imagines that he is going to grow to a full height like any other boy, and wishes to save the world when he's "tall enough". He will become a vegetable in a few years, and simply a spirit some time after that. His parents set their concerned eyes on him, and cry at his hopeless future.

DMD Information Site. 11 Jan. 2000. 11 Nov. 2005. .